SAB1408565

Anti-Dystrophin Antibody

rabbit polyclonal

• Synonim(y): BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269
• Kod UNSPSC: 12352203
• NACRES: NA.41

Właściwości

• product name: Anti-DMD antibody produced in rabbit, purified immunoglobulin, buffered aqueous solution
• pochodzenie biologiczne: rabbit
• Poziom jakości: 100
• białko sprzężone: unconjugated
• forma przeciwciała: purified immunoglobulin
• rodzaj przeciwciała: primary antibodies
• klon: polyclonal
• Postać: buffered aqueous solution
• masa cząsteczkowa: antigen ~72.2 kDa
• reaktywność gatunkowa: human
• metody: western blot: 1 μg/mL
• numer dostępu NCBI: NM_004016.1
• numer dostępu UniProt: P11532
• Warunki transportu: dry ice
• temp. przechowywania: −20°C
• docelowa modyfikacja potranslacyjna: unmodified
• informacje o genach: human ... DMD(1756)

Opis

Opis ogólny

The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. (provided by RefSeq)

Immunogen

DMD (NP_004007.1, 1 a.a. ~ 635 a.a) full-length human protein.

Sequence
MREQLKGHETQTTCWDHPKMTELYQSLADLNNVRFSAYRTAMKLRRLQKALCLDLLSLSAACDALDQHNLKQNDQPMDILQIINCLTTIYDRLEQEHNNLVNVPLCVDMCLNWLLNVYDTGRTGRIRVLSFKTGIISLCKAHLEDKYRYLFKQVASSTGFCDQRRLGLLLHDSIQIPRQLGEVASFGGSNIEPSVRSCFQFANNKPEIEAALFLDWMRLEPQSMVWLPVLHRVAAAETAKHQAKCNICKECPIIGFRYRSLKHFNYDICQSCFFSGRVAKGHKMHYPMVEYCTPTTSGEDVRDFAKVLKNKFRTKRYFAKHPRMGYLPVQTVLEGDNMETPVTLINFWPVDSAPASSPQLSHDDTHSRIEHYASRLAEMENSNGSYLNDSISPNESIDDEHLLIQHYCQSLNQDSPLSQPRSPAQILISLESEERGELERILADLEEENRNLQAEYDRLKQQHEHKGLSPLPSPPEMMPTSPQSPRDAELIAEAKLLRQHKGRLEARMQILEDHNKQLESQLHRLRQLLEQPQAEAKVNGTTVSSPSTSLQRSDSSQPMLLRVVGSQTSDSMGEEDLLSPPQDTSTGLEEVMEQLNNSFPSSRGHNVGSLFHMADDLGRAMESLVSVMTDEEGAE

Postać fizyczna

Solution in phosphate buffered saline, pH 7.4

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Informacja o środkach bezpieczeństwa

• Kod klasy składowania: 10 - Combustible liquids
• Klasa zagrożenia wodnego (WGK): WGK 1
• Temperatura zapłonu (°F): Not applicable
• Temperatura zapłonu (°C): Not applicable